Article
Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1.
The Journal of cell biology - 10 Jun 2002
Meriin Anatoli B, Zhang Xiaoqian, He Xiangwei, Newnam Gary P, Chernoff Yury O, Sherman Michael Y
Abstract excerpt
The cause of Huntington's disease is expansion of polyglutamine (polyQ) domain in huntingtin, which makes this protein both neurotoxic and aggregation prone. Here we developed the first yeast model, which establishes a direct link between aggregation of expanded polyQ domain and its cytotoxicity. Our data indicated that deficiencies in molecular chaperones Sis1 and Hsp104 inhibited seeding of polyQ aggregates,...
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